It is always hard for me to know how to start these updates because rarely is it good news that I share. It has been a busy few months. I didn’t write an official June update after the ALS clinic day and so I guess there is a lot to update. 

A quick refresh from our June appointment: 

• His ALSFRS score has gone down to 34 out of 48. He’s averaging losing 5 points every 3 months. 
• His MIP score (lungs related) and it dropped from 75 to 58. Anything under 60 qualifies him for a Bipap machine via insurance and so he started using it at night two weeks ago.  It helps give his diaphragm a break while he sleeps so ideally it is more capable during his wake hours.
• We also went through the steps to get him an Eye Gaze machine (essentially a laptop that has a camera on it that makes it so he can use his eyes to control it like you would a mouse).
• We also setup a subscription for protein shakes through our insurance as they want him to be drinking two of them per day to help keep his calorie count up and maintain his weight. 
• We tested out some large sized utensils but it’s easier for him to actually use regular silverware. 
• He got a neck collar as his neck strength is going but it makes it tiring for his arms to hold his phone up to see to be able to communicate so he hasn’t used it much. We got him an airplane type pillow he often uses in the evening while watching TV. 
• They also set him up with a suction machine which can help for things like brushing teeth and spitting out liquid but he hasn’t found the need to use it yet. 

Fun things lately:

• Adeline attended VBS at our church for the first time and LOVED it! I volunteered to take photos and help with crafts while both Grandma’s took turns hanging out with Elizabeth and then she joined me the last day. It was a great week! 
• I had a training day with new team members joining our team and working their way up. We brought on 2 new first shooters who will be training up to shoot weddings solo next year and also two additional girls that are in a training period and then they will move up to second shooters with the goal of first shooters in the next 1-1.5 years. Going into 2024 we will have 6 first shooters and 2 second shooters as well as an assistant and myself! 
• Our new home is almost complete! We did the final walk through this week and get the keys on the 10th. We cannot wait!
• Dirk quit working in mid-July and has been slowly adjusting to life without work. We threw him a little retirement party which was Hawaii themed and some of the family dressed up as his past jobs. We catered in Chipotle and I made tons of homemade frozen yogurt for a Menchie’s style frozen yogurt bar.
• Lots of time spent out in the garden in the girls with water toys, baking, thrift store shopping, picking fruits and veggies, playing games and just having fun soaking up our last week living with Grandma and Grandpa. 

Update on Rebecca: 

It has been a very busy and exhausting few months. We are in peak wedding season for me right now which included me personally shooting 5 weddings this past month with a total of 16 weddings photographed by me and my team combined in July. I’ve been both very physically and emotionally exhausted. I’ve also been battling shoulder and back pain as well as nerve issues in my hand/arm which has resulted in going to the chiropractor 3x per week. I’ve come to realize that times when I am physically exhausted, I am much more prone to struggle emotionally as well. I am looking forward to wedding season starting to slow down in terms of me shooting as my daily household tasks increase. We made the decision for me personally to take time off of personally photographing weddings after this season for the time being. My associate team however is growing and will continue to shoot weddings next year.

Talking to Adeline:

After Adeline starting to express more concern and questions regarding how Dirk has been changing, we officially sat down and had a more formal conversation with her. We explained he has something called ALS which is essentially causing his body to become old faster. We opened the door to allowing her to ask any questions she might have whenever she thinks of them and she asked a few in regards to what she sees – why Daddy talks different, why his hands look different, why he can’t pick her up anymore…etc. We didn’t talk about death directly but we did tell her that he will not get better and he will continue to get worse. She handled it like she does everything, amazingly and like a 5-year-old. We don’t want to break her innocence by introducing concepts to her that she wouldn’t quite comprehend but did open the door that she can express her concerns and questions whenever she wants. And she has continued to ask questions. We would appreciate prayers as these conversations continue to develop.

How is Dirk progressing? 
His communication is almost all via swiping out messages into an app on his phone. We can understand simple in-context verbal phrases but for the most part he uses his app to talk. I imagine once he gets the EyeGaze machine we will work on programming often used phrases and he will start using that more to communicate. He can still eat and drink just fine. He occasionally coughs on water and rarely on food but is still able to feed himself and eat enough to maintain his weight. It just takes him a while to get through a meal due to slow hands and mouth. We do cut things for him to save him time and we have to be diligent about making sure he is getting high calorie meals and he supplements with protein shakes. 

He is still mobile and can walk around the house fine, although slowly. When going outside he uses a cane. Stairs are getting tougher but he is still able to climb them as long as there is a handrail. He gets tired quickly. Things like showering or getting dressed are exhausting. His range of motion is decreasing and says he feels very stiff. His hands are stiff, fingers are starting to curl and struggling to do anything detail oriented. His neck muscles are going which results in his head looking towards the ground when tired and neck braces are hot and uncomfortable. Everything is simply…hard. It takes a lot of energy for him to do anything and so he gets tired or frustrated quickly. However, he still cracks jokes, laughs often and is in good spirits. He has his bad days, as well all do, but I am amazed at his resilience and upbeat nature though all of this. 

Everyday seems to be trying to re-learn how to do something together or communicate. I am always overly trying to help Dirk even when he doesn’t need it and his daily life is slowly becoming more frustrating. Communication is hard and slow. It is a constant battle both physically and emotionally for us both. 

I would argue that this is where it starts to get hard. 

Prayers:

• Prayers for guidance in answering Adelines questions.
• We are moving next week!! Prayers for a good transition and wisdom in how we outfit the house to be more friendly for Dirk. 
• Energy and endurance for Rebecca as she is in peak wedding season, moving and alllll the things.
• That his progression would slow down. 
• Wisdom as we potentially seek out some other treatment options.
• Prayers that he would continue to avoid falling or getting injured.
• Continued prayers that his lungs would stay healthy and he will not aspirate food or drinks.

A few praises:

• My parents sold in less than a week after listing! 
• When me and the girls have gotten sick recently, Dirk hasn’t!!
• Our house was built in only four months!! 

As always, some photos: